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Abstract Background The histopathological analysis of minor salivary gland biopsies, particularly through the quantification of the Focus Score (FS), is pivotal in the diagnostic workflow for Sjögren's Syndrome (SS). AI-based image recognition using deep learning models has demonstrated potential in enhancing diagnostic accuracy and efficiency in preclinical research. Objectives The primary aim of this investigation was to utilize an auto-machine learning (autoML) platform for the automated segmentation and quantification of FS on histopathological slides, aiming to augment diagnostic precision and speed in SS. Methods A cohort comprising 86 patients with sicca syndrome (37 diagnosed with SS based on the 2016 ACR/EULAR Classification Criteria and 49 non-SS) was selected for an in-depth histological examination. A repository of 172 slides (two per patient) was assembled, encompassing 74 slides meeting the classificatory thresholds for SS (FS ≥ 1, indicative of lymphocytic infiltration) and 98 slides showcasing normal salivary gland histology. The autoML platform utilized (Giotto, L2F, Lausanne Switzerland) employed a Convolutional Neural Network (CNN) architecture (ResNet-152) for the training and validation phases, using a dataset of 172 slides. Results The developed model exhibited a reliability score of 0.88, proficiently distinguishing SS cases, with a sensitivity of 89.47% (95% CI: 66.86% to 98.70%) and a specificity of 88.24% (95% CI: 63.56% to 98.54%). The model found histological slides of suboptimal quality (e.g., those compromised during fixation or staining processes) to be the most challenging for accurate classification. Conclusion AutoML platforms offer a rapid and flexible approach to developing machine learning models, even with smaller datasets, as demonstrated in this study for SS. These platforms hold significant potential for enhancing diagnostic precision and efficiency in both clinical and research settings. Multicentric studies with larger patient cohorts are essential for thorough evaluation and validation of this innovative diagnostic approach.

This literature review looks at Sjogren's Syndrome (SS), a chronic autoimmune disorder affecting exocrine glands, particularly the lacrimal and salivary glands. SS manifests as ocular and oral dryness, with severe complications like visual dysfunction and corneal perforation, as well as systemic implications, such as interstitial lung disease and lymphoma. This review explores the use of tear film biomarkers to diagnose SS, emphasizing the significance of their identification in aiding clinical diagnosis and differentiation from other diseases. This study identified and analyzed 15 papers, encompassing 1142 patients and employing various tear sample collection methods. Tear biomarkers were categorized by function and explored in-depth. Categories include (1) antimicrobials, antivirals, and antifungals; (2) components of immune regulation; (3) components that regulate metabolic processes; and (4) inflammatory markers. Noteworthy findings include the potential diagnostic values of tear lysozyme, lactoferrin, dinucleoside polyphosphates, cathepsin, defensin, antibodies, epidermal fatty acid-binding protein, HLA-DR, ADAM10, aquaporin 5, and various miRNAs and mRNAs. Overall, our understanding of SS tear film composition is enhanced, providing valuable insights into the pathogenesis of SS and offering a foundation for future diagnostic and therapeutic advancements in autoimmune conditions affecting the ocular surface. [ABSTRACT FROM AUTHOR]

La maladie de Sjögren est une affection auto-immune complexe qui persiste à poser d'importants défis malgré les progrès de la recherche. Il s'agit d'une maladie rare dont la prévalence est estimée entre 0,01 % et 0,05 %. Son incidence est variable selon les pays mais pourrait être en augmentation sur les deux dernières décennies. Les facteurs de risque, incluant les antécédents familiaux, le stress et les expositions hormonales, demeurent incomplètement élucidés. La maladie de Sjögren peut coexister avec d'autres maladies auto-immunes en particulier avec les thyroïdites auto-immunes, la polyarthrite rhumatoïde, le lupus érythémateux systémique et la sclérodermie systémique. C'est la maladie auto-immune au cours de laquelle l'augmentation du risque de lymphome est la plus importante. Il pourrait également y avoir, comme au cours d'autres maladies auto-immunes telles que la polyarthrite rhumatoïde ou le lupus, une augmentation du risque cardiovasculaire, bien que les preuves à ce sujet restent à consolider. Les recherches actuelles révèlent l'existence de divers sous-groupes de patients, définis par leurs symptômes, leurs paramètres clinico-biologiques, et/ou leur origine géographique. Cette variabilité est à l'origine de profils évolutifs différents et d'un risque de mortalité très fortement influencé par la présence ou non de lymphome, la survenue ce dernier étant particulièrement lié à l'activité systémique de la maladie. Cette revue générale abordera les bases épidémiologiques, l'hétérogénie phénotypique et pronostique soulignant la nécessité d'une approche thérapeutique individualisée au cours de la maladie de Sjögren. Sjögren disease is a complex autoimmune disorder with ongoing challenges despite research advances. It is a rare disease with an estimated prevalence between 0.01% and 0.05%. Its incidence varies by country but could be increasing over the last two decades. Risk factors, including family history, stress, and hormonal exposures, remain incompletely understood. Sjögren disease can coexist with other autoimmune diseases, particularly autoimmune thyroid disease, rheumatoid arthritis, systemic lupus erythematosus, and systemic sclerosis. It is the autoimmune disease with the highest increased risk of lymphoma. There may also be, as in other autoimmune diseases such as rheumatoid arthritis or lupus, an increased risk of cardiovascular disease, although evidence remains to be consolidated. Current research reveals the existence of various patient subgroups, defined by their symptoms, clinical presentation, biological parameters, and/or geographic origin. This variability leads to different evolutionary profiles and a risk of mortality strongly influenced by the presence or absence of lymphoma, the occurrence of which is particularly linked to the systemic activity of the disease. This general review will address the epidemiological foundations, phenotypic and prognostic heterogeneity, highlighting the need for an individualized therapeutic approach in Sjögren disease. [ABSTRACT FROM AUTHOR]

Tertiary lymphoid structures (TLSs) are formed in tissues targeted by chronic inflammation processes, such as infection and autoimmunity. In Sjögren's disease, the organization of immune cells into TLS is an important part of disease progression. Here, we investigated the dynamics of tissue resident macrophages in the induction and expansion of salivary gland TLS. We induced Sjögren's disease by cannulation of the submandibular glands of C57BL/6J mice with LucAdV5. In salivary gland tissues from these mice, we analyzed the different macrophage populations prior to cannulation on day 0 and on day 2, 5, 8,16 and 23 post-infection using multicolored flow cytometry, mRNA gene analysis, and histological evaluation of tissue specific macrophages. The histological localization of macrophages in the LucAdV5 induced inflamed salivary glands was compared to salivary glands of NZBW/F1 lupus prone mice, a spontaneous mouse model of Sjögren's disease. The evaluation of the dynamics and changes in macrophage phenotype revealed that the podoplanin (PDPN) expressing CX3CR1+ macrophage population was increased in the salivary gland tissue during LucAdV5 induced inflammation. This PDPN+ CX3CR1+ macrophage population was, together with PDPN+CD206+ macrophages, observed to be localized in the parenchyma during the acute inflammation phase as well as surrounding the TLS structure in the later stages of inflammation. This suggests a dual role of tissue resident macrophages, contributing to both proinflammatory and anti-inflammatory processes, as well as their possible interactions with other immune cells within the inflamed tissue. These macrophages may be involved with lymphoid neogenesis, which is associated with disease severity and progression. In conclusion, our study substantiates the involvement of proinflammatory and regulatory macrophages in autoimmune pathology and underlines the possible multifaceted functions of macrophages in lymphoid cell organization. [ABSTRACT FROM AUTHOR]

Tertiary lymphoid structures (TLSs) are formed in tissues targeted by chronic inflammation processes, such as infection and autoimmunity. In Sjögren’s disease, the organization of immune cells into TLS is an important part of disease progression. Here, we investigated the dynamics of tissue resident macrophages in the induction and expansion of salivary gland TLS. We induced Sjögren’s disease by cannulation of the submandibular glands of C57BL/6J mice with LucAdV5. In salivary gland tissues from these mice, we analyzed the different macrophage populations prior to cannulation on day 0 and on day 2, 5, 8, 16 and 23 post-infection using multicolored flow cytometry, mRNA gene analysis, and histological evaluation of tissue specific macrophages. The histological localization of macrophages in the LucAdV5 induced inflamed salivary glands was compared to salivary glands of NZBW/F1 lupus prone mice, a spontaneous mouse model of Sjögren’s disease. The evaluation of the dynamics and changes in macrophage phenotype revealed that the podoplanin (PDPN) expressing CX3CR1+ macrophage population was increased in the salivary gland tissue during LucAdV5 induced inflammation. This PDPN+ CX3CR1+ macrophage population was, together with PDPN+CD206+ macrophages, observed to be localized in the parenchyma during the acute inflammation phase as well as surrounding the TLS structure in the later stages of inflammation. This suggests a dual role of tissue resident macrophages, contributing to both proinflammatory and anti-inflammatory processes, as well as their possible interactions with other immune cells within the inflamed tissue. These macrophages may be involved with lymphoid neogenesis, which is associated with disease severity and progression. In conclusion, our study substantiates the involvement of proinflammatory and regulatory macrophages in autoimmune pathology and underlines the possible multifaceted functions of macrophages in lymphoid cell organization.

BACKGROUND: Pulmonary disease is a potentially serious yet underdiagnosed complication of Sjögrens syndrome, the second most common autoimmune rheumatic disease. Approximately 16% of patients with Sjögrens demonstrate pulmonary involvement with higher mortality and lower quality of life. RESEARCH QUESTION: Clinical practice guidelines for pulmonary manifestations of Sjögrens were developed by the Sjögrens Foundation after identifying a critical need for early diagnosis and improved quality and consistency of care. STUDY DESIGN AND METHODS: A rigorous and transparent methodology was followed according to American College of Rheumatology guidelines. The Pulmonary Topic Review Group (TRG) developed clinical questions in the PICO (Patient, Intervention, Comparison, Outcome) format and selected literature search parameters. Each article was reviewed by a minimum of two TRG members for eligibility and assessment of quality of evidence and strength of recommendation. Guidelines were then drafted based on available evidence, expert opinion, and clinical importance. Draft recommendations with a clinical rationale and data extraction tables were submitted to a Consensus Expert Panel for consideration and approval, with at least 75% agreement required for individual recommendations to be included in the final version. RESULTS: The literature search revealed 1,192 articles, of which 150 qualified for consideration in guideline development. Of the original 85 PICO questions posed by the TRG, 52 recommendations were generated. These were then reviewed by the Consensus Expert Panel and 52 recommendations were finalized, with a mean agreement of 97.71% (range, 79%-100%). The recommendations span topics of evaluating Sjögrens patients for pulmonary manifestations and assessing, managing, and treating upper and lower airway disease, interstitial lung disease, and lymphoproliferative disease. INTERPRETATION: Clinical practice guidelines for pulmonary manifestations in Sjögrens will improve early identification, evaluation, and uniformity of care by primary care physicians, rheumatologists, and pulmonologists. Additionally, opportunities for future research are identified.

Introduction: Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease characterized by a chronic grade of inflammation. Cardiovascular events represent the major causes of morbidity and mortality in patients with inflammatory rheumatic diseases; however, the significance and prevalence of cardiovascular disease in patients with pSS remain unclear. Objective: To determine the clinical significance of cardiovascular disease in pSS and analyze the risk of cardiovascular disease according to glandular/extraglandular involvement and positivity to anti-Ro/SSA and/or anti-La/SSB autoantibodies. Methods: A retrospective study including patients diagnosed with pSS fulfilling the 2016 ACR/EULAR classification criteria was followed and evaluated in our outpatient clinic between 2000 and 2022. The prevalence of cardiovascular risk factors with pSS was evaluated, and a possible association with clinical and immunological characteristics, the treatments received, and the impact on cardiovascular disease were determined. Univariate and multivariate regression analyses were performed in an attempt to determine potential risk factors associated with cardiovascular involvement. Results: A total of 102 pSS patients were included. Eighty-two percent were female, with a mean age of 65±24 years and a disease duration of 12.5 ±6 years. Thirty-six patients (36%) had at least one cardiovascular risk factor. Arterial hypertension was diagnosed in 60 (59%) patients, dyslipidemia in 28 (27%), diabetes in 15 (15%), obesity in 22 (22%), and hyperuricemia in 19 (18%). History of arrhythmia was found in 25 (25%), conduction defects in 10 (10%), arterial peripheral vascular disease in 7 (7%), venous thrombosis in 10 (10%), coronary artery disease in 24 (24%), and cerebrovascular disease in 22 (22%) of patients. Patients with extraglandular involvement had a higher prevalence of arterial hypertension (p=0.04), dyslipidemia (p=0.003), LDL mean values (p=0.038), hyperuricemia (p=0.03), and coronary artery disease (p=0.01) after adjusting for age, sex, disease duration, and the significant variables in the univariate analysis. Patients with Ro/SSA and La/SSB autoantibodies had a substantially higher risk of hyperuricemia (p=0.01), arrhythmia (p=0.01), coronary artery disease (p=0.02), cerebrovascular disease (p=0.02), and venous thrombosis (p =0.03). In the multivariate logistic regression analysis, higher odds of cardiovascular risk factors were associated with extraglandular involvement (p=0.02), treatment with corticosteroids (p=0.02), ESSDAI>13 (p=0.02), inflammatory markers including ESR levels (p 0.007), and serologic markers such as low C3 levels (p=0.03) and hypergammaglobulinemia (p=0.02). Conclusions: Extraglandular involvement was associated with a higher prevalence of arterial hypertension, dyslipidemia, hyperuricemia, and coronary artery disease. Anti-Ro/SSA and anti-La/SSB seropositivity was associated with a higher prevalence of cardiac rhythm abnormalities, hyperuricemia, venous thrombosis, coronary artery disease, and cerebrovascular disease. Raised inflammatory markers, disease activity measured by ESSDAI, extraglandular involvement, serologic markers including hypergammaglobulinemia and low C3, and treatment with corticosteroids were associated with a higher risk for cardiovascular comorbidities. Key Points • Patients with pSS are vulnerable to cardiovascular risk factors. There is an interconnection between extraglandular involvement, disease activity, inflammatory markers, and cardiovascular risk comorbidities. • Anti-Ro/SSA and anti-La/SSB seropositivity was associated with a higher frequency of cardiac conduction abnormalities, coronary artery disease, venous thrombosis, and stroke. • Hypergammaglobulinemia, elevated ESR, and low C3 are associated with a higher prevalence of cardiovascular comorbidities. • Valid risk stratification tools to help with prevention and consensus on the management of CVDs in pSS patients are warranted. [ABSTRACT FROM AUTHOR]

Objetivo: describir los hallazgos ecográficos en las glándulas salivares para el diagnóstico del síndrome de Sjögren primario. Metodología: se realizó una revisión narrativa de la literatura, con búsqueda en bases de datos seleccionando los principales artículos de revisión e investigaciones originales en español e inglés publicados en los últimos 20 años. Resultados: los hallazgos confirman el valor diagnóstico de la ecografía como estudio no invasivo de las glándulas salivares. Conclusión: la ecografía de glándulas salivares es un método útil y confiable para el diagnóstico del síndrome de Sjögren.

Objective: The BETY-Biopsychosocial Questionnaire (BETY-BQ) is a scale developed to assess the biopsychosocial domains of patients with rheumatic diseases under a single roof. The study aimed to determine the validity and reliability of the BETY-BQ in patients with primary Sjögren's syndrome (pSS)., Methods: At enrollment and 1 week, 91 patients with pSS completed the BETY-BQ. Construct validity was measured by correlating the BETY-BQ total score responses with the Health Assessment Questionnaire, Hospital Anxiety and Depression Scale, 36-Item Short-Form Health Survey, EULAR Sjögren's Syndrome Patient-Reported Index, EULAR Sjögren's Syndrome Disease Activity Index, Primary Sjögren's Syndrome Quality of Life questionnaire, and Euro-QoL 5D., Results: The BETY-BQ correlated high to moderate with Health Assessment Questionnaire, bodily pain subscale of 36-Item Short-Form Health Survey, Euro-Qol 5D, Primary Sjögren's Syndrome Quality of Life questionnaire, Hospital Anxiety and Depression Scale, and EULAR Sjögren's Syndrome Patient-Reported Index (0.776 to 0.557, P < .05). Spearman's correlation coefficients between BETY-BQ total scores at baseline and average 1 week were very high (rho = 0.98, P < .001) and indicated substantial agreement between test-retest scores (intraclass correlation coefficient = 0.99, P < .001). Internal consistency reliability at baseline was 0.91 for the BETY-BQ., Conclusions: BETY-BQ is valid and reliable for assessing biopsychosocial status in patients with pSS and can be used to measure outcomes in pSS., (© Japan College of Rheumatology 2024. Published by Oxford University Press. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site–for further information please contact journals.permissions@oup.com.)

Purpose: To report increased corneal bioavailability of allogenic serum when used in combinations with Therapeutic Hyper-CL™ soft contact lens in a patient with severe Sjögren's syndrome-associated dry eye. Observations: A 57-year-old woman with a medical history of bilateral severe Sjögren's syndrome-associated dry eye and previous amniotic membrane patch for autoimmune corneal perforation in her left eye was referred for left eye recurrence of progressive melting and pending perforation. After manual corneal trephination, full thickness transplant and sutured amniotic membrane patch, a Therapeutic Hyper-CL™ soft contact lens (EyeYon Medical, Ness Tziona, Israel) was fit. The patient was commenced in the left eye with topical corticosteroid, antibiotic, and allogenic serum eye drops. In the right eye the patient had silicone hydrogel bandage contact lens and was under same treatment of the left eye for previous endothelial keratoplasty. In order to evaluate the efficacy and increased corneal availability of drugs provided by Therapeutic Hyper-CL™ compared with silicone hydrogel soft contact lens, anterior segment OCT was performed. Conclusions and importance: The anterior segment OCT showed a thicker meniscus of fluid and possibly subsequent increase of trophic factors bioavailability in left eye compared with right eye. Therefore, in case of severe and refractory dry-eye disease the combination of Therapeutic Hyper-CL™ and serum eye drops may be representing a valid therapeutic approach.

Sjögren's syndrome (SjS) is a heterogeneous systemic disease. The abnormal responses to La/SSB and Ro/SSA of both B-cells and T-cells are implicated as well as others, in the destruction of the epithelium of the exocrine glands, whose tissue characteristically shows a peri-epithelial lymphocytic infiltration that can vary from sicca syndrome to systemic disease and lymphoma. Despite the appearance of new autoantibodies, anti-Ro/SSA is still the only autoantibody included in the American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria and is used extensively as a traditional biomarker in clinical practice. The study and findings of new autoantibodies in SjS has risen in the previous decade, with a central role given to diagnosis and elucidating new aspects of SjS physiopathology, while raising the opportunity to establish clinical phenotypes with the goal of predicting long-term complications. In this paper, we critically review the classic and the novel autoantibodies in SjS, analyzing the methods employed for detection, the pathogenic role and the wide spectrum of clinical phenotypes. [ABSTRACT FROM AUTHOR]

Sjögren’s syndrome (SjS) is a heterogeneous systemic disease. The abnormal responses to La/SSB and Ro/SSA of both B-cells and T-cells are implicated as well as others, in the destruction of the epithelium of the exocrine glands, whose tissue characteristically shows a peri-epithelial lymphocytic infiltration that can vary from sicca syndrome to systemic disease and lymphoma. Despite the appearance of new autoantibodies, anti-Ro/SSA is still the only autoantibody included in the American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria and is used extensively as a traditional biomarker in clinical practice. The study and findings of new autoantibodies in SjS has risen in the previous decade, with a central role given to diagnosis and elucidating new aspects of SjS physiopathology, while raising the opportunity to establish clinical phenotypes with the goal of predicting long-term complications. In this paper, we critically review the classic and the novel autoantibodies in SjS, analyzing the methods employed for detection, the pathogenic role and the wide spectrum of clinical phenotypes.

• Objective clinical, histological and US manifestations of sicca syndrome are highly frequent in SSc patients. • Sjogren-SSc patients (overlap) had more severe glandular involvement than isolated Sjogren and isolated Sicca-SSc patients. • Specific SSc US patterns of salivary gland involvement are still to be defined. This study aimed to characterize the whole phenotype of Systemic sclerosis (SSc) patients with sicca symptoms, using major salivary glands Ultrasound (SGUS) parameters, minor salivary glands biopsies (mSGB) and clinical findings, and to compare these characteristics with those from patients with Sjogren's Disease (SjD), and patients with sicca manifestations from other causes. Sixty SSc patients fulfilling the 2013 ACR/EULAR classification criteria and with subjective self-declared sicca symptoms were consecutively recruited and had SGUS and mSGB. Fifteen SSc patients without subjective sicca symptoms and 65 patients with sicca symptoms from other causes (including 37 SjD with no SSc). SSc patients with subjective sicca symptoms had frequent objective clinical (up to 83 %), histological (44 % of Focus score≥1/ mm2) and US anomalies (63 % of OMERACT ≥2). 53 % patients without subjective clinical complaint also had abnormal objective tests, suggesting the existence of a sub clinical involvement of salivary glands in SSc. SjD-SSc patients had more severe glandular involvement as compared to patients with isolated SjD and isolated Sicca-SSc patients (70%, 48,6 % and 38% of patients with OMERACT ≥2 respectively) suggesting additive impact of both diseases on glandular physiology and structure. SjD-SSc overlap have more severe sicca features as compared to isolated sicca-SSc and isolated SjD, suggesting a specific impact of SSc on salivary gland physiology. Further translational studies are needed to identify the underlying pathways that could serve as therapeutic targets. [ABSTRACT FROM AUTHOR]

The management of connective tissue diseases is dramatically evolving with the advent of biologics and novel oral systemic therapeutics. Despite involvement in the care of these complex patients, there is a knowledge gap in the field of dermatology regarding these emerging agents. The first article in this continuing medical education series discusses new and emerging therapeutics for lupus erythematosus and Sjögren syndrome that target cells, intracellular signaling pathways, and cytokines. [ABSTRACT FROM AUTHOR]

• Les atteintes systémiques du Sjögren sont multiples et de gravité différente. • L'ESSDAI est un index regroupant les atteintes systémiques les plus fréquentes et les plus quantifiables et ses items font partie du score d'évaluation thérapeutique appelé STAR. • Le lymphome est la complication la plus redoutée. • La tubulopathie entraîne rarement une altération de la fonction rénale mais nécessite une surveillance. • La neuronopathie/ganglionopathie peut avoir un retentissement fonctionnel majeur. La maladie de Sjögren (MS) ou syndrome de Sjögren primitif (SSp) peut s'accompagner de manifestations systémiques. Il s'agit des atteintes indépendantes de l'exocrinopathie, qui peuvent être très sévères, souvent en lien avec une hyperactivation lymphocytaire B ou la présence d'une cryoglobuline. La fréquence des atteintes systémiques est diversement évaluée en fonction des pays d'origine, de la durée d'évolution du Sjögren ou des méthodes de détection utilisées, mais elles concernent globalement 20 à 70 % des patients. On peut dichotomiser les atteintes systémiques en 2 types : celles entraînant un risque majeur pour le patient, ou au pronostic fonctionnel défavorable, que sont les lymphomes, les vascularites cryoglobulinémiques, les atteintes du système nerveux central, les ganglionopathies et les glomérulonéphrites prolifératives, et celles considérées comme d'évolution moins sévère. Cependant la mortalité globale est identique à la population générale. Les manifestations systémiques les plus fréquentes et mesurables ont été regroupées au sein d'un score consensuel international : l' EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI), utilisé dans les essais cliniques, et récemment incorporées dans l'index STAR (Sjogren's Tool for Assessing Response). Primary Sjögren syndrome (pSS) is a progressive autoimmune disease characterized by sicca and systemic manifestations that are frequent and potentially severe, especially when related to B cell activity or the presence of cryoglobulinemia, but the pathological role of the anti-SSA antibodies has never been demonstrated. The frequency of the systemic manifestations in pSS is differentially evaluated related to disease duration or the methods used to detect them, but they are supposed to concern 20 to 70% of the patients. The systemic manifestations can be artificially divided in two types: the life-threatening manifestations (lymphoma, vasculitis with cryoglobulinemia, central nervous system involvement, ganglioneuronopathy and membranoproliferative glomerulonephritis) and the others manifestations. However, the mortality rate in pSS is similar to the general population. The more frequent and easy to evaluate systemic manifestations have been gathered in the ESSDAI score (EULAR Sjogren's Syndrome Disease Activity Index), an international consensual score used in clinical trials and recently incorporated in the STAR index (Sjogren's Tool for Assessing Response). [ABSTRACT FROM AUTHOR]

Sjögren syndrome (SS) is an autoimmune inflammatory disorder characterized by secretory dysfunction in the eye and mouth; in the eye, this results in tear film instability, reduced tear production, and corneal barrier disruption. A growing number of studies show that homeostasis of the ocular surface is impacted by the intestinal microbiome, and several 16S sequencing studies have demonstrated dysbiosis of the intestinal microbiota in SS patients. In this study, we utilized metagenomic sequencing to perform a deeper analysis of the intestinal microbiome using stools collected from sex- and age-matched healthy (n = 20), dry eye (n = 4) and SS (n = 7) subjects. The observed Operational Taxonomic Units (OTUs) and Shannon alpha diversity were significantly decreased in SS compared to healthy controls, and there was a significant inverse correlation between observed OTUs and ocular severity score. We also identified specific bacterial strains that are differentially modulated in SS vs. healthy subjects. To investigate if the differential composition of intestinal microbiome would have an impact on the immune and eye phenotype, we performed functional studies using germ-free mice colonized with human intestinal microbiota from SS patients and healthy controls. Flow cytometry analysis demonstrated reduced frequency of CD4+ FOXP3+ cells in ocular draining cervical lymph nodes (CLN) in mice colonized with SS patient intestinal microbiota 4 weeks post-colonization. We also found that offspring of SS-humanized mice also have fewer CD4+FOXP3+ cells in the CLN as well as spleen, demonstrating vertical transmission. SS-humanized mice subjected to desiccating stress exhibited greater corneal barrier disruption as compared to healthy control humanized mice under the same conditions. Taken together, these data support the hypothesis that the intestinal microbiota can modulate ocular surface health, possibly by influencing development of CD4+ FOXP3+ regulatory T cells (Tregs) in the ocular draining lymph nodes.

Background: The Sjogren syndrome (SS) is the least well-known rheumatic condition. We aim to gradually resolve it by standardizing the process of SS care in our country, at first for primary Sjogren syndrome cases, through developing Iran Primary Sjogren Syndrome Registry (IRAPSS) which will create a representative and comparable data bank., Methods: Guilan Primary Sjogren Syndrome Registry (GUIRAPSS) is a pilot phase of IRAPSS, which is a prospective cohort. Care of its patients is done based on EULAR Sjögren's syndrome disease activity index (ESSDAI) recommendations. Other IRAPSS outcome measurements are EULAR Sjogren syndrome patient reported index (ESSPRI) and Sjögren's syndrome disease damage index (SSDDI) for detecting disease damage., Results: Female-male ratio was 17.5. The age at expert diagnosis was 46.97 ± 11.93 years old. The most common comorbidity was hypothyroidism (28.38%), followed by musculoskeletal conditions (27.02%). 28.38% had fatigue. Active disease existed based on the clinical form of ESSDAI and ESSDAI, 31.8% and 81.8% respectively. The most involved organs during disease activity based on ESSDAI were biological 68.12% following pulmonary (12.16%) and respiratory (10.81%) system. Hydroxychloroquine was the most prescribed drug (72.97%), followed by prednisolone (28.38%)., Conclusion: Disease registries provide an ideal opportunity for gathering standardized and comparable data which provides needed items for creating, updating, or adapting pSS classification criteria/diagnosis, outcome measurements and or treatment guidelines., Competing Interests: The authors declare that they have no competing interests., (© 2024 Iran University of Medical Sciences.)

Background: Pulmonary disease is a potentially serious yet underdiagnosed complication of Sjögren's syndrome, the second most common autoimmune rheumatic disease. Approximately 16% of patients with Sjögren's demonstrate pulmonary involvement with higher mortality and lower quality of life. Research question: Clinical practice guidelines for pulmonary manifestations of Sjögren's were developed by the Sjögren's Foundation after identifying a critical need for early diagnosis and improved quality and consistency of care. Study design: and methods: A rigorous and transparent method-ology was followed according to American College of Rheumatology guidelines. The Pulmonary Topic Review Group (TRG) developed clinical questions in the PICO (Patient, Intervention, Comparison, Outcome) format and selected literature search parameters. Each article was reviewed by a minimum of two TRG members for eligibility and assessment of quality of evidence and strength of recommendation. Guidelines were then drafted based on available evidence, expert opinion, and clinical importance. Draft recommendations with a clinical rationale and data extraction tables were submitted to a Consensus Expert Panel for consideration and approval, with at least 75% agreement required for individual recommendations to be included in the final version. Results: The literature search revealed 1,192 articles, of which 150 qualified for consideration in guideline development. Of the original 85 PICO questions posed by the TRG, 52 recommendations were generated. These were then reviewed by the Consensus Expert Panel and 52 recommendations were finalized, with a mean agreement of 97.71% (range, 79%-100%). The recommendations span topics of evaluating Sjögren's patients for pulmonary manifestations and assessing, managing, and treating upper and lower airway disease, interstitial lung disease, and lymphoproliferative disease. Interpretation: Clinical practice guidelines for pulmonary manifestations in Sjögren's will improve early identification, evaluation, and uniformity of care by primary care physicians, rheumatologists, and pulmonologists. Additionally, opportunities for future research are identified. [ABSTRACT FROM AUTHOR]

Ataxia is a frequent symptom in neurological cases with many causes. Sensory ataxia (due to involvement of the proprioceptive pathways) is observed in conditions affecting the central nervous system (spinal cord disorder) and the peripheral nervous system (peripheral neuropathy). The latter correspond to what we refer to as 'ataxic neuropathies'. Ataxic neuropathies represent a wide and heterogeneous spectrum of disorders that may affect dorsal root nerves, dorsal root ganglia, nerve trunks, distal nerve endings or all of them together. The identification of a predominant sensory ataxia in a case of peripheral neuropathy should raise the possibility of some specific etiologies. We propose here to present the main causes of ataxic neuropathies, which are identified with diagnostic workflows that are dictated by the topography of the likely sites of lesions in the proprioceptive pathway together with the timing of their occurrence (acute, subacute, or chronic). [ABSTRACT FROM AUTHOR]

Primary Sjögren’s syndrome (pSS) is a progressive autoimmune disease and is characterized by eye and mouth dryness due to lymphocytic infiltration in lacrimal and salivary glands leading to tissue destruction, but it can also present systemic manifestations including lung involvement. Respiratory manifestations in pSS have a prevalence of 9-20% and can be due to airway and/or lung parenchyma involvement, such as in particular in interstitial lung diseases like lymphocytic interstitial pneumonia (LIP). LIP is an inflammatory diffuse parenchymal lung disease, which is almost invariably associated with other conditions, such as autoimmune diseases and immunodeficiency states, and usually affects women with a mean age of 50 years. We described a case of patient with LIP who was referred to our Internal Medicine Unit and the diagnostic issues related to the patient age and comorbidities.

Primary Sjögren’s syndrome (pSS) is an autoimmune disease which primarily affects the exocrine glands, but can also affect other organs, including the nervous system. Many studies have reported evidence of autonomic nervous system (ANS) dysfunction in pSS which may contribute to a wide range of symptoms and functional burden. Symptoms of ANS dysfunction are common and widespread among patients with pSS and are associated with other features of the disease, particularly fatigue. Accumulating data on the inter-relationship between the ANS and the immune system via the vagus nerve have been reported. Vagus nerve stimulation (VNS) has also been associated with improvement in fatigue in patients with pSS. Taken together, these data suggest that the ANS may be a potential treatment target for pSS, in particularly those with fatigue being a predominant symptom. Future research to dissect the link between the ANS, immune dysregulation and clinical manifestations in pSS and to evaluate the potential of VNS as a therapy for pSS is warranted.

Background: This study addresses the challenging characterisation and differentiation of CIDP versus CIDP in association with Sjögren's syndrome to facilitate the process in clinical routine. Methods: Patients with both CIDP and Sjögren's syndrome and CIDP without Sjögren's syndrome were compared concerning relevant differences in clinical, laboratory and electrophysiological findings. 154 patients who fulfilled the diagnostic EFNS/PNS criteria for CIDP were included in the analysis. 54 of these patients additionally fulfilled the ACR/EULAR classification criteria for Sjögren's syndrome. Results: The frequency of female patients was higher in patients with CIDP and Sjögren's syndrome (52%) versus CIDP patients without Sjögren's syndrome (28%). Furthermore, the occurrence of cranial nerve impairment was significantly higher in patients with Sjögren's syndrome (39% versus 14%). There were no significant group differences in the evaluation of initial symptoms, severity of disability judged by INCAT disability scale score, presence or distribution of sensory deficits, limb weakness and the presence of ataxia, pain or dysautonomia, CSF laboratory or electrophysiological findings. Conclusions: In conclusion, our data indicate that cranial nerve impairment and female gender might represent red flags for an additional Sjögren's syndrome in patients with CIDP. The patterns of clinical disabilities and electrophysiological findings due to peripheral nerve damage are similar in both CIDP entities. [ABSTRACT FROM AUTHOR]

Primary Sjögren's syndrome (pSS) is an autoimmune disease which primarily affects the exocrine glands, but can also affect other organs, including the nervous system. Many studies have reported evidence of autonomic nervous system (ANS) dysfunction in pSS which may contribute to a wide range of symptoms and functional burden. Symptoms of ANS dysfunction are common and widespread among patients with pSS and are associated with other features of the disease, particularly fatigue. Accumulating data on the inter-relationship between the ANS and the immune system via the vagus nerve have been reported. Vagus nerve stimulation (VNS) has also been associated with improvement in fatigue in patients with pSS. Taken together, these data suggest that the ANS may be a potential treatment target for pSS, in particularly those with fatigue being a predominant symptom. Future research to dissect the link between the ANS, immune dysregulation and clinical manifestations in pSS and to evaluate the potential of VNS as a therapy for pSS is warranted. [ABSTRACT FROM AUTHOR]

Copyright of Journal of Ankara University Faculty of Medicine / Ankara Üniversitesi Tip Fakültesi Mecmuasi is the property of Galenos Yayinevi Tic. LTD. STI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Objectives: Anti-Ro60 and anti-Ro52 autoantibodies are frequently used as diagnostic biomarkers for Sjogren's disease, but their clinical significance in systemic lupus erythematosus (SLE) is not well characterised., Methods: Patients fulfilling SLE classification criteria were studied according to their anti-Ro status. We defined Ro positivity (Ro+) as those who have either anti-Ro60 or anti-Ro52 positivity. Patient characteristics and disease outcomes, including High Disease Activity Status (HDAS) defined as an ever attainment of SLEDAI2K ≥10, adjusted mean SLEDAI (AMS), and time-adjusted mean clinical SLEDAI (excluding serologic activities) were compared using linear or logistic regressions. Furthermore, isolated or dual positivity of anti-Ro60 and anti-Ro52 were studied., Results: Out of 409 patients, 47.2% were Ro+. Ro+ patients were predominantly Asian, had positive dsDNA and hypocomplementemia. They showed a higher likelihood of HDAS (OR 1.65, 95% CI 1.10-2.48, p= 0.015), AMS > 4 (OR 1.84, 1.18-2.88, p= 0.007), and more frequent use of glucocorticoids (OR 1.87, 1.16-3.03, p= 0.011) and immunosuppressants (OR 2.0, 1.26-3.17, p= 0.003). Additionally, 24.4% of Ro+ patients experienced sicca symptoms, and hypergammaglobulinemia was significantly more common. Multivariate analysis confirmed that Asian ethnicity, severe flares, AMS, hypocomplementemia, rheumatoid factor, proteinuria, leucopenia, and sicca symptoms were significantly linked to Ro positivity., Conclusion: Anti-Ro positivity is associated with higher disease activity and increased treatment needs. Ro positivity correlates with laboratory abnormalities such as hypocomplementemia and leucopenia. These findings highlight the importance of anti-Ro60/Ro52 testing in the clinical evaluation of SLE., (© The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology.)

Helicobacter pylori (H. pylori) is a gram-negative bacterium that adapts to the gastric mucosa and provokes symptoms associated with gastritis. Chronic H. pylori infection in patients with a genetic predisposition can trigger autoimmune diseases due to the immune interaction of cellular and humoral responses. Infections are a triggering factor for systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and Sjögren syndrome (SS), although the association between H. pylori and these diseases is unclear. Therefore, we reviewed this interaction and its clinical importance.

BackgroundANCA-associated vasculitis (AAV) and Sjögren’s syndrome (SS) are uncommon autoimmune diseases. The co-occurrence in the same patient has been rarely described. Acromegaly has been associated with autoimmune thyroiditis, but the prevalence of other autoimmune disorders such as AAV and SS has not been evaluated in acromegaly.MethodsCharacterization of a patient with acromegaly and two rare autoimmune diseases—SS and AAV (microscopic polyangiitis (MPA))—by autoantibody-array and whole exome sequencing (WES). Single-center retrospective review of medical records of acromegaly patients to explore the prevalence of diagnosed autoimmune diseases.ResultsWe report a Caucasian woman in her 50’s with a serologically (anti-SSA/Ro, anti-MPO-ANCA antibodies) and histologically confirmed diagnosis of symptomatic SS and MPA. SS with MPO-ANCA positivity preceded MPA. An exploratory autoantigen array detected a broad spectrum of autoantibodies. WES revealed heterozygous carrier status of the PTPN22 mutation R620W, which is associated with an increased risk for autoimmunity. A similar combination of positive anti-SSA/Ro autoantibodies and ANCA was only present in 5/1184 (0.42%) other patients tested for both antibodies in our clinic over six years. Amongst 85 acromegaly patients seen at our clinic in a 20-year period, 12% had a clinically relevant associated immunological disease.ConclusionWe present a rare case of SS and AAV in a patient with acromegaly and multiple autoantibody specificities. Patients with SS and ANCA should be closely monitored for the development of (subclinical) AAV. Whether acromegaly represents a risk for autoimmunity should be further investigated in prospective acromegaly cohorts.

Objective: Sjögren syndrome is a systemic inflammatory disease causing gland dysfunction. Few (and contradictory) reports on the mucosal effects of Sjögren syndrome have appeared. Here, we objectively demonstrate nasal dryness in Sjögren syndrome patients and explore the effect of such dryness on olfaction. Methods: Thirty‐four consecutive patients with primary Sjögren syndrome were enrolled in this cross‐sectional study. The control group consisted of 21 age‐ and sex‐matched volunteers. Medical histories and nasal findings were recorded. The Connecticut Chemosensory Clinical Research Center test was used to evaluate olfactory function. All subjects underwent mucucociliary clearance analysis (the saccharin test and peak nasal inspiratory flowmetry). The intranasal Schirmer test was used to evaluate the nasal cavity. Results: The nasal Schirmer test scores were 8.4 mm (right) and 8 mm (left) (P =.041, P =.016, respectively, compared to controls). The Chi‐squared test revealed significant differences (compared to controls) in nasal dryness (P =.001), postnasal drip (P =.04), and smell (a decrease) (P =.005). Neither olfactory function nor mucociliary clearance differed between the groups. We noted a trend toward a positive correlation between olfactory function and the nasal Schirmer score but statistical significance was not attained. Conclusion: The intranasal Schirmer test objectively showed that Sjögren syndrome patients exhibited nasal cavity dryness; this is useful in terms of follow‐up. This did not affect olfactory function. Level of Evidence: 4 Laryngoscope, 131:370–373, 2021 [ABSTRACT FROM AUTHOR]

Background: ANCA-associated vasculitis (AAV) and Sjögren's syndrome (SS) are uncommon autoimmune diseases. The co-occurrence in the same patient has been rarely described. Acromegaly has been associated with autoimmune thyroiditis, but the prevalence of other autoimmune disorders such as AAV and SS has not been evaluated in acromegaly. Methods: Characterization of a patient with acromegaly and two rare autoimmune diseases—SS and AAV (microscopic polyangiitis (MPA))—by autoantibody-array and whole exome sequencing (WES). Single-center retrospective review of medical records of acromegaly patients to explore the prevalence of diagnosed autoimmune diseases. Results: We report a Caucasian woman in her 50's with a serologically (anti-SSA/Ro, anti-MPO-ANCA antibodies) and histologically confirmed diagnosis of symptomatic SS and MPA. SS with MPO-ANCA positivity preceded MPA. An exploratory autoantigen array detected a broad spectrum of autoantibodies. WES revealed heterozygous carrier status of the PTPN22 mutation R620W, which is associated with an increased risk for autoimmunity. A similar combination of positive anti-SSA/Ro autoantibodies and ANCA was only present in 5/1184 (0.42%) other patients tested for both antibodies in our clinic over six years. Amongst 85 acromegaly patients seen at our clinic in a 20-year period, 12% had a clinically relevant associated immunological disease. Conclusion: We present a rare case of SS and AAV in a patient with acromegaly and multiple autoantibody specificities. Patients with SS and ANCA should be closely monitored for the development of (subclinical) AAV. Whether acromegaly represents a risk for autoimmunity should be further investigated in prospective acromegaly cohorts. [ABSTRACT FROM AUTHOR]

Copyright of Revista Sociedad Colombiana de Oftalmología is the property of Sociedad Colombiana de Oftalmologia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Objectives: Sjögren syndrome (SS) is considered as a major etiologic factor for primary salivary gland lymphoma (SGL). However, the percentage of SGL that is caused by SS (and thus the real impact of SS on SGL epidemiology) is unclear. We aimed to assess the prevalence of SS in patients with SGL through a systematic review and meta-analysis.Methods: Electronic databases were searched for studies assessing the presence of SS in patients with SGL. Pooled prevalence of SS in SGL was calculated, with a subgroup analysis based on histotype (mucosa-associated lymphoid tissue [MALT] vs non-MALT).Results: Sixteen studies with 665 SGLs were included. Pooled prevalence of SS in SGL was 18.2%, with high heterogeneity among studies. In MALT SGL, the prevalence of SS was 29.5%, with moderate heterogeneity. In non-MALT SGL, the prevalence of SS was 0%, with null heterogeneity.Conclusions: SS seems to be responsible for a significant but minor portion of SGLs. SS appears involved in MALT-type SGL but not in other histotypes. [ABSTRACT FROM AUTHOR]

Background and Objective: Hyposalivation and xerostomia can result from a variety of conditions. Diagnosis is based on a combination of medical history, clinical and serological parameters, imaging, and minor salivary gland biopsy when indicated. The Objective was to characterize microscopic changes in minor salivary gland biopsies taken in patients with xerostomia. Materials and Methods: 10-year retrospective analysis of minor salivary gland biopsies, 2007–2017. Histomorphometric analysis included gland architecture, fibrosis, fat replacement, inflammation and stains for IgG/IgG4, when relevant. Results: 64 consecutive biopsies, of which 54 had sufficient tissue for diagnosis of Sjogren’s Syndrome (SS) were included (18 males, 46 females, average age 56 (±12.5) years). Only 12 (22.2%) were microscopically consistent with SS, none stained for IgG4. Medical conditions were recorded in 40 (63%), most frequently hypertension and hyperlipidemia (28% each). Medications were used by 45 (70%), of which in 50% more than one. Xerostomia in non-SS cases was supported by abnormal gland morphology, including acinar atrophy, fibrosis and fatty replacement. All morphological abnormalities are correlated with age, while fatty replacement correlated with abnormal lipid metabolism. Multiple medications correlated with microscopic features which did not correspond with SS. Conclusions: SS was confirmed in a minority of cases, while in the majority fatty replacement, fibrosis and multiple medications can explain xerostomia, and are related to aging and medical conditions. Medical history and auxiliary tests could lead to correct diagnosis in non-SS patients, avoiding biopsy. The necessity of a diagnostic biopsy should be given serious consideration only after all other diagnostic modalities have been employed.

Correction: In the publication study period was missed. Study period "from 1st June 2017 to 30th November 2017" has been added. Introduction: Dry eye is a disorder of the tear film which occurs due to tear deficiency or excessive tear evaporation. It causes damage to the interpalpebral ocular surface and is associated with a variety of symptoms reflecting ocular discomfort. Materials and Methods: This was a hospital-based prospective observational study conducted to study the demography of dry eye patients and associated attributable risk factors for the disease. Every patient presenting with symptoms suggestive of dry eye was given a questionnaire, as per Salisbury Eye Evaluation Study and thorough ocular examination was done along with two tests for dry eye i.e Schirmer test I measuring less than 10mm and Tear film break up time measuring less than 10 sec. Final data analysis was done using statistical tool SPSS. Results: Demographic profile revealed a female preponderance in the patients with a ratio of 1.9:1. Amongst them, prevalence of premenopausal age was 53%. Dry eye was found to be more common in age group between 30 to 40 years (29%). The modifiable attributable risk factors were urban residence (67%), exposure to air condition and computer user (52%), indoor workers (59%), and environmental pollutants (60%). Conclusions: This study revealed the patients who presented with dry eye were mostly female of premenopausal age group, and exposed to modifiable risk factors which included residing at urban areas, indoor work, and exposure to environmental pollutants.

Neuromyelitis Optic (NMO) is an inflammatory disorder involving central nervous system which often co-exists with other autoimmune diseases such as Sjögren’s syndrome (SS). NMO manifestation could precede or follow SS, but the role of anti-SSA in the pathogenesis of NMO remains unclear. We present a case of NMO with anti-AQP4 anti-SSA antibody positive. A-44-year-old female presented with right side weakness. The symptoms began with numbness that improved spontaneously. She also complained pain and dry sensations on her eyes. Schirmer test on her left eye, antinuclear antibody (ANA) and anti- SSA antibody were positive. Cervical MRI revealed intramedullary lesion on T2- weighted-image at C2-C5 level. She was diagnosed as NMO with positive anti- AQP4 and probable SS. She received 1g methylprednisolone for 5 days proceeded with mycophenolic acid. One-year observation showed clinical improvement. Systemic autoantibodies must substantially be evaluated in NMO. Comprehensive diagnosis and providing appropriate immunosuppressant might prevent further disability and relapse.

Objectives: Polyneuropathy is the most common neurological complication in primary Sjögren's syndrome (pSS). A ratio of sural nerve and superficial radial nerve sensorial action potential amplitudes (SRARs) of <0.4 is an indicator for early axonal neuropathy. We evaluated the polyneuropathies and SRARs in pSS patients. Method: Fifty-two female patients who were diagnosed with pSS according to the European-American Consensus Criteria and 45 healthy controls were enrolled. Nerve conduction studies were performed to diagnose polyneuropathy. Sensory axonal polyneuropathy was diagnosed in three patients, so SRARs were compared in 49 patients and 50 healthy controls. Results: Fifty-two patients with pSS underwent nerve conduction tests. The sural sensory nerve action potential (SNAP) was <6 µV in threepatients and they were diagnosed with sensory axonal neuropathy. SRARs were evaluated in 49 female patients, with a mean age of 51.98 ± 10.79 years and 50 healthy controls with a mean age of 50.52 ± 12.55 years. The mean disease duration was 7.59 ± 6.17 years. The SRAR values were different between the patient and control groups. SRAR was <0.4 in 20.4% of the patient group and <0.4 in 6% of the control group. The SRAR value was not statistically different within the patient group based on anti-Ro and anti-La. Discussion: The potential for neurological involvement in patients with pSS who have no signs or injury should be evaluated because nervous system involvement in pSS is a negative prognostic factor. SRAR in patients with pSS can be used as a marker for the early detection of axonal neuropathy. [ABSTRACT FROM AUTHOR]

Purpose: Inner ear involvement has been reported in systemic rheumatic disease while detection of cochlin-specific antibodies has been reported in patients with idiopatic sensorineural hearing loss, suggesting cochlin's strong link to autoimmune hearing loss. The aim of this cross-sectional study was to calculate the prevalence of sensorineural hearing loss (SNHL) in patients with systemic rheumatic diseases, and to investigate any potential correlation with human antibodies to cochlin. Methods: Patients with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), Sjogren's syndrome (SS) and systemic sclerosis (SSc) according to the criteria of American College of Rheumatology were included in the study. All patients underwent a complete ear-nose-throat physical examination and audiological evaluation with pure tone audiometry and impedance audiometry. Pure tone average was calculated, taking as a starting point the hearing loss in dB according to the recommendation 02/1 of "Bureau International d' Audiophonologie" (BIAP) so as an average hearing threshold value. Sera of all patients were tested for the presence of IgG antibodies to human cochline (COCH-IgG). Sex and age-matched healthy subjects were included as controls to each group. Results: A total of 133 patients were studied; 60 with RA, 41 with SLE, 24 with SS and 8 with SSc. 61.4% of patients reported vertigo, 41% hyperacousis, 39% hearing loss, 38% tinnitus, 37.9% headache and 2.1% sensation of ear pressure with unremarkable otoscopy. The prevalence of SNHL calculated for patients affected by RA, SLE, SS and SSc was 66.6%, 31.71%, 54.17%, and 75% respectively. The calculated average hearing thresholds value in RA was increased in comparison to SLE (p < 0.05). In addition it was also higher in patients with RA and secondary SS, in comparison to RA patients (p > 0.05). There was statistically significant correlation of average hearing threshold with disease activity score 28 (DAS28) in RA, but no correlation observed with disease activity index (SLEDAI) in SLE. COCH-IgG antibodies were detected in only two samples. The results were compared with those of their respective sex and age-matched healthy subjects. Conclusion: Our study revealed increased prevalence of SNHL in patients with systemic autoimmune rheumatic disease but no correlation of hearing loss with COCHIgG antibodies. The mechanism of inner ear damage remains unknown; thus, additional prospective studies will be needed to elucidate its pathogenesis. [ABSTRACT FROM AUTHOR]

The triad of central nervous system symptoms, visual disturbance and hearing impairment is an oft-encountered clinical scenario. A number of immune-mediated diseases should be considered among the differential diagnoses including: Susac syndrome, Cogan syndrome or Vogt-Koyanagi-Harada disease; demyelinating conditions such as multiple sclerosis or neuromyelitis optica spectrum disorder; systemic diseases such as systemic lupus erythematosus, Sjögren syndrome or Behcet disease and granulomatous diseases such as sarcoidosis. In this article, we coin the term 'BEE syndromes' to draw attention to the various immune-mediated diseases that affect the brain, eye and ear. We present common disease manifestations and identify key clinical and investigation features. [ABSTRACT FROM AUTHOR]

Purpose: Primary Sjogren syndrome (PSS) is an autoimmune disease characterized by symptoms of a dry mouth and eyes, in which other organs and systems are widely involved. Central nervous system (CNS) involvement in PSS is reported in a wide range between 2.5–60%. The reason is that the clinical picture can remain asymptomatic despite the presence of CNS involvement in the disease process. In this study, our aim was to evaluate subclinical cognitive impairment in patients with PSS by investigating P300 potential parameters. Method: Forty-three female patients with PSS (mean age: 52.6 ± 11.4 years) and 35 healthy female controls (mean age: 54.5 ± 8.09 years) were included in the study. Mini-Mental State evaluations (MMSE) and brain MRI were performed in the patient and control groups. An event-related evoked potentials test (P300) was applied to those with normal MMSE. Results: The P300 latencies of patients with PSS were significantly longer compared with the control group (p =.019). In patients with PSS, there was no difference in P300 parameters between ANA, Anti-SSA, Anti-SSB-positive and negative patients, and patients with or without sedimentation and CRP elevation. In addition, brain MRI revealed no statistically significant difference between patients with PSS with and without ischemic gliotic lesions (p=.48). Conclusion: In our study, P300 latency was also found to be significantly longer in patients who had no white matter change. We believe that prolonged P300 potential latencies without associated white matter lesions in brain imaging may be associated with subclinical CNS involvement. [ABSTRACT FROM AUTHOR]

El ojo seco es la manifestación clínica de una disfunción de la unidad funcional lagrimal que resulta en una inestabilidad de la película lagrimal, con hiperosmolaridad, irritación crónica e inflamación de la superficie ocular. Enfermedades sistémicas, como la Enfermedad de Sjögren, el Lupus Eritematoso Sistémico y la Artritis Reumatoide están frecuentemente asociadas al Síndrome del Ojo Seco. El objetivo principal de su tratamiento debe ser el mejorar la calidad de vida, reducir las molestias del paciente y las alteraciones de la superficie ocular. El manejo de esta patología puede ser conservador o invasivo, dependiendo de la severidad de la misma. La primera línea de tratamiento es la sustitución de la lágrima deficiente con lubricantes artificiales en lágrimas y geles. En los casos moderados el uso de lágrimas artificiales sin preservantes y la terapia antiinflamatoria tópica, son las terapias recomendadas. La oclusión temporal o permanente de los conductos lagrimales, el uso de lentes de contacto terapéuticos o cámaras humidificadoras constituyen otras opciones terapéuticas para el síndrome del ojo seco moderado. En los casos graves pueden ser útiles la aplicación tópica de suero antólogo, la terapia antiinflamatoria sistémica, sustitución androgénica, ciclosporina, secretagogos y los procedimientos quirúrgicos

Primary Sjögren syndrome (pS) is associated with autoantibodies such as rheumatoid factor (RF) and anti-nuclear antibodies such as anti-Ro (SS-A) and/or La (SS-B). Recent developments within autoimmune diagnostics allow quantitation of RF subclasses and anti-Ro epitopes. Will this refinement by autoimmune diagnostics help predicting development of extraglandular manifestations (EGM) in pS patients? A cohort of pS and rheumatoid arthritis (RA) patients with keratoconjunctivitis sicca (n = 35 and 16, resp) was included. Of the pS patients, 54% developed one or more EGM. Antibodies quantitated were IgM-RF, IgA-RF, IgG-RF, anti-Ro52, and anti-Ro60. Upon analysis of RF isotypes, pS patients generally displayed higher IgA-RF concentrations than RA patients (126 versus 49 U/ml, p = 0.015), while the dominant RF isotype in RA patients was IgM-RF (82.5 versus 38 U/ml, p = 0.012). No differences were observed regarding IgG-RF concentrations. In pS without/with EGM, the median RF IgM concentrations were similar, while RF IgA and IgG concentrations tended to be lower in pS patients with EGM > 1. Both Ro epitopes were strongly recognized by almost all pS patients, independent from EGM, while these antibodies were absent in RA patients. Primary Sjögren syndrome and RA patients have distinct serological profiles when analysing RF and Ro-specific antibodies. A longitudinal study of switched RF isotypes in pS patients is worthwhile from an immunological point of view, but its value is limited regarding identification of pS patients prone to developing EGM or RA patients prone to developing secondary sicca symptoms. [ABSTRACT FROM AUTHOR]

Connective tissue diseases (CTDs) such as systemic lupus erythematosus, systemic sclerosis, myositis, Sjögren's syndrome, and rheumatoid arthritis are systemic diseases which are often associated with a challenge in diagnosis. Autoantibodies (AAbs) can be detected in these diseases and help clinicians in their diagnosis. Actually, pathophysiology of these diseases is associated with the presence of antinuclear antibodies. In the last decades, many new antibodies were discovered, but their implication in pathogenesis of CTDs remains unclear. Furthermore, the classification of these AAbs is nowadays misused, as their targets can be localized outside of the nuclear compartment. Interestingly, in most cases, each antibody is associated with a specific phenotype in CTDs and therefore help in better defining either the disease subtypes or diseases activity and outcome. Because of recent progresses in their detection and in the comprehension of their pathogenesis implication in CTD-associated antibodies, clinicians should pay attention to the presence of these different AAbs to improve patient's management. In this review, we propose to focus on the different phenotypes and features associated with each autoantibody used in clinical practice in those CTDs. [ABSTRACT FROM AUTHOR]

El Síndrome de Sjögren (SS) es una patología inflamatoria crónica, infradiagnosticada y de etiología desconocida, que se caracteriza por la infiltración linfocitaria de las glándulas exocrinas. La sintomatología puede ser muy variada. Inicialmente, lo más común es que se presente sequedad de mucosas (principalmente oculares y bucales); también se puede asociar a otras enfermedades autoinmunes, y hasta en el 65% de los casos, se pueden presentar manifestaciones extraglandulares, pudiéndose afectar cualquier parte del organismo. En muchos casos, la sintomatología inicial es leve, achacándose habitualmente a un posible disconfort en el puesto de trabajo. A esto hay que añadir que a nivel laboral no existe literatura específica en la que poder basarse para realizar una adecuada valoración médico-laboral. El objetivo de este trabajo ha sido crear una herramienta que englobe, por una parte, la vigilancia de la salud específica, y por otra, definir (aunque sea de forma orientativa), los criterios para poder realizar una correcta valoración de la capacidad laboral en los trabajadores con Síndrome de Sjögren.

Giriş: Primer Sjögren sendromunda (pSS) başlıca bulgular ağız ve göz kuruluğu olmakla birlikte birçok organ sisteminde tutulum görülebilmektedir. Akciğer ve hematolojik tutulumlar pSS'nin bilinen ekstraglandüler tutulumlarındandır. Burada pSS, intersitisyel akciğer hastalığı (İAH) ve miyelodisplastik sendrom (MDS) beraberliği olan bir olguyu paylaşmak istedik. Olgu: Kuru öksürük ve efor dispnesi ile başvurduğu dış merkezde yapılan tetkiklerine İAH tespit edilen hasta romatolojik yönden araştırılmak üzere tarafımıza başvurdu. İAH yönünden ek risk faktörü ve COVID-19 öyküsü bulunmayan hastanın romatolojik sorgusunda sikka semptomları dışında anlamlı semptom yoktu. Fizik muayenede hasta soluk ve kaşektik görünümdeydi, sol posterior servikalde LAP ve akciğerde ralleri vardı. Laboratuvar tetkiklerinde bisitopeni (Hg: 8,9 gr/dL, Plt: 17,0000, BK: 2,600) mevcuttu. ESR >140 mm/st, CRP: 50 mg/L, RF:16 ANA 1/3200 granüler, anti-SSA +3, anti-SSB ve anti-Ro52 +1 pozitif, ANCA IFA ve PR3/MPO ELISA negatif, C3-C4 ve immünoglobulin G, A, M ve G alt tipleri normal sınırlarda, anti-CCP negatif saptandı. Schirmer testi pozitif, tükrük bezi biyopsisi Chisolm-Mason evre 0'dı. HRCT'de her iki akciğerde periferik alanlarda fibrotik bantlar, bal peteği görünümleri ve yer yer buzlu cam alanları mevcuttu (Resim 1). SJS ve İAH tanısı konan hasta, LAP ve bisitopeni yönünden hematolojiye yönlendirildi. Kemik iliği biyopsisinde ile miyeloid seride maturasyonda duraksama saptandı ve hastaya desitabin tedavisi başlandı. Takipte pulmoner semptomları spontan gerileyen hastaya immünosüpresif tedavi başlanmadı. Ek semptomu olmayan hastaya 6. ayda kontrol HRCT planlanarak takibe alındı. Sonuç: pSS'de akciğer tutulumu %10-20 arasındadır. Sıklıkla NSIP paterni görülmekle beraber LIP pSS için spesifiktir. pSS hastalarında %62'ye varan oranda çeşitli hematolojik tutulumlar izlenebilir. Sitopeniler, hipo/hipergamaglobulinemi, kryoglobulinemi, monoklonal gamopati ve lenfoma bilinen hematolojik tutulumlardır. MDS pSS seyrinde sık görülen bir antite olmamakla beraber otoimmün fenomenin bir parçası olarak romatolojik hastalıkların seyrinde ortaya çıkabileceği bildirilmiştir. pSS'de farklı organ sistemleri aynı anda etkilenebilir, tedavi planlanırken tüm tutulumlar göz önünde bulundurularak tedavi bireyselleştirilmelidir. Biz de atipik ve farklı komplikasyonların birliktelik gösterdiği olgumuzda bu noktaya dikkat çekmek istedik. [ABSTRACT FROM AUTHOR]

Objectives: The aim of this study was to make an initial estimation on the effects of a sodium bicarbonate and xylitol spray (Cariex® ), associated with non-surgical periodontal therapy, in participants with primary Sjögren's syndrome.Background: Sjögren's syndrome (SS) is a multisystem autoimmune disease that predominantly involves salivary and lachrymal glands, with the clinical effect of dry eyes and mouth.Materials and Methods: A prospective cohort of 22 women and two men has been evaluated. They were randomized into three groups (eight patients each): Group A) those treated once with non-surgical periodontal therapy, education and motivation to oral hygiene, associated with the use of Cariex® ; Group B) treated only with Cariex® ; Group C) treated only with non-surgical periodontal therapy, education and motivation to oral hygiene. Clinical variables described after treatment were unstimulated whole salivary flow, stimulated whole salivary flow, salivary pH, reported pain (using Visual Analogue Scale) and the Periodontal Screening and Recording index.Results: Salivary flow rate improved in all groups, but the difference was statistically significant only in those treated with Cariex® , alone or in combination with periodontal therapy. Gingival status improved in participants who underwent periodontal non-surgical therapy while remained unchanged in those only treated with Cariex® . Reported pain decreased in all groups, showing the best result in participants treated with periodontal therapy together with Cariex® .Conclusions: We propose a practical approach for improving gingival conditions and alleviating oral symptoms in patients with SS. Future randomized and controlled trials are however required to confirm these results as well as larger population, and also assessing other parameters due to oral dryness, possible oral infections and more comprehensive periodontal indices. [ABSTRACT FROM AUTHOR]

Introduction: Sjögren’s syndrome (SS) is a chronic inflammatory auto-immune disease of the exocrine glands which incidence increases with age. Sex ratio is 9.9 female for 1 male. Observation: A 67-year-old female patient presented for xerostomia leading to major functional impairment. The medical history of the patient was pulmonary sarcoidosis, with join and adenoid involvement, considered as cured; left partial parotidectomy, with a diagnosis of acinous carcinoma, followed 7 years later by a total parotidectomy due to tumor recurrence, and followed by external radiotherapy (70 Gy). Arterial hypertension, hypothyroïditis and diabetes mellitus were also noticed. She reported xerostomia and xerophtalmia. Seric anti-SSA antibodies were positive and histologic findings were compatible with SS. Commentaries: Despite other co-morbidities, the diagnosis was hidden by previous parotidectomy and cervical radiotherapy. Thus, SS should not be underdiagnosed in patients with complex medical history.